Pulmonary atresia/critical stenosis with intact ventricular septum: prediction of outcome in the second trimester of pregnancy

Abstract

Objectives To determine which cardiac parameters provide the best prediction of postnatal outcome—biventricular (BV) versus non‐BV—in fetuses with pulmonary atresia/critical stenosis with intact ventricular septum (PA/CS‐IVS). Methods We searched our database for cases of PA/CS‐IVS prenatally diagnosed in 2001–2009. Only fetuses diagnosed ⩽28 weeks were included. Data of 16 fetuses (nine PA, seven CS) were retrieved and analyzed. Receiver‐operating characteristics curves were constructed to assess the sensitivity and specificity of cardiac features for predicting postnatal outcome. Results Twelve fetuses had a BV outcome and four had non‐BV repair. Cut‐off values yielding the best results for a non‐BV outcome were a tricuspid valve/mitral valve ratio ⩽0.83, a pulmonary valve/aortic valve ratio ⩽0.75, tricuspid inflow duration/cardiac cycle length ⩽36.5%, and a right ventricle/left ventricle length ratio ⩽0.64. If 3/4 markers are present, this predicts a non‐BV outcome with sensitivity of 100% and specificity of 92%, and both are 100% if all the four criteria are fulfilled. Conclusions The postnatal outcome of fetuses with PA‐CS/IVS can be predicted in the second trimester at the first echocardiography by a four‐criterion scoring system. This is clinically relevant since it allows early selection of candidates for fetal intervention and early and precise parental counseling. Copyright © 2011 John Wiley & Sons, Ltd.